Abstract
Endocrine pancreatic tumors (EPTs) are rare entities with a low incidence (3-10 per million). A relatively frequent feature (15-53%) among this group of tumors is represented by the non-functioning endocrine pancreatic tumors (NFEPTs) whose peculiarity is due to the absent secrection of mature or active hormones, leaving the patient free from clinically evident hypersecrection syndromes, generally discovered when the mass effect becomes evident, the adjacent pancreatic structures (splenic, superior mesenteric and portal vein, celiac or superior mesenteric arteries, common bile duct, duodenum, etc.) are infiltrated or hepatic metastases are growing. A potentially malignant attitude is high and well related to the dimension of the tumor with inexorably fatal outcome if appropriate surgery is delayed. The size of the mass and an evident involvement of nearer vascular structures might rise some doubts about the decision to radically remove the tumor. An aggressive surgery should be balanced with the risk/benefit ratio for generally young patients with a reasonable long life expectancy. We parallel two clinical cases among the patients we observed through the years at our institution and operated on by the same surgeon, who were displaying the same tumoral histology and loco-regional invasiveness of portomesenteric axis, but differing one each other for the presence of metastatic disease to the liver the first case. Further aim of the present report is to support the evidence of the feasibility and safeness of extensive surgical demolition with prosthetic reconstruction of the porto-mesenteric axis.
Highlights
Endocrine pancreatic tumors (EPTs) are rare entities with a low incidence (3-10 per million) [1], being the 5% of all neoplastic variety of pancreas [2].A relatively frequent feature (15-53%) among this group of tumors is represented by the non-functioning endocrine pancreatic tumors (NFEPTs) [3,4] with an absent secrection of mature or active hormones, and no clinically evident hypersecrection syndromes
A recent prospective study showed that the rate of NFPETs in MEN1 patients is higher than 55% when endoscopic ultrasound (EUS) is performed to detect them [7]
Since the number and size of the NFPETs tend to increase in time and the affected population is relatively young, this rate is certainly going to grow as time passes by
Summary
Endocrine pancreatic tumors (EPTs) are rare entities with a low incidence (3-10 per million) [1], being the 5% of all neoplastic variety of pancreas [2]. A relatively frequent feature (15-53%) among this group of tumors is represented by the non-functioning endocrine pancreatic tumors (NFEPTs) [3,4] with an absent secrection of mature or active hormones, and no clinically evident hypersecrection syndromes. The consequences are represented by indolent clinical courses and late diagnoses so they are generally discovered when the mass effect becomes evident, the adjacent pancreatic structures are infiltrated or hepatic metastases are growing. The size of the mass and an evident involvement of nearer vascular structures might raise some doubts about the decision to radically remove the tumor along with infiltred major vessels and the subsequent need of their reconstruction. Further aim is to support the evidence of the feasibility and safeness of extensive surgical demolition with prosthetic reconstruction of the porto-mesenteric axis
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